Xeroderma pigmentosum (XP) is a highly complex autosomal recessive disease linked to an enzymatic DNA repair disorder. Herein, we report the clinical and evolutionary aspects of XP in Niger. Materials and Methods: Our study included patients diagnosed with XP from two families. Results: We collected eight patients with an average age of 5.5 years, with extremes of two and thirteen years. The sex ratio was 1. Consanguinity was found in both families. The first tumor appeared between three and five years of age in six cases and around eight years of age in two cases. The tumors were cutaneous in seven cases, and extra-cutaneous in five cases. Histology made it possible to identify basal cell and squamous cell carcinomas without any case of melanoma. Five out of eight died between the age of eight and twelve years. Conclusion: Prevention through the reduction of new cases by genetic counseling and antenatal diagnosis in families at risk is necessary.

XP; Basal cell carcinoma; Squamous cell carcinomas; Early death; Niamey